Eosinophilic Granulomatosis With Polyangiitis (Egpa)

(Churg-Strauss syndrome)

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) is a systemic, small and medium vessels necrotizing vasculitis, characterized by extravascular granulomas, eosinophilia and tissue infiltration due eosinophils. It occurs in people with asthma in adulthood, allergic rhinitis, nasal polyps, or a combination. The diagnosis is best confirmed by biopsy. Treatment is mainly with corticosteroids and in severe disease with additional immunosuppressive drugs.

Eosinophilic granulomatosis with polyangiitis (EGPA) occurs to a million people in approximately 3 minutes. the average age of onset is 48 years.

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) is a systemic, small and medium vessels necrotizing vasculitis, characterized by extravascular granulomas, eosinophilia and tissue infiltration due eosinophils. It occurs in people with asthma in adulthood, allergic rhinitis, nasal polyps, or a combination. The diagnosis is best confirmed by biopsy. Treatment is mainly with corticosteroids and in severe disease with additional immunosuppressive drugs. Eosinophilic granulomatosis with polyangiitis (EGPA) occurs to a million people in approximately 3 minutes. the average age of onset is 48 years. EGPA is characterized by necrotizing granulomas extravascular (usually rich in eosinophils), eosinophilia, and tissue infiltration by eosinophils. However, these anomalies do not always exist simultaneously. The vasculitis typically affects small and medium-sized arteries. Each institution may be affected, but lung, skin, nose, cardiovascular system, kidneys, peripheral nervous system, central nervous system, joints and gastrointestinal tract are most often affected. Occasionally the Capillaritis can cause pulmonary alveolar hemorrhage. Etiology The cause of EGPA is unknown. However, an allergic mechanism is involved in which the tissue is directly damaged by eosinophils and degranulation of neutrophils. The activation of T lymphocytes seems to contribute to the maintenance of eosinophilic inflammation. The syndrome occurs in patients who show asthma starting in adulthood, allergic rhinitis, nasal polyps, or a combination thereof. Antineutrophil cytoplasmic autoantibodies (ANCA) of cases are present in about 40%. Symptoms and complaints The syndrome has three phases which may overlap: prodromal: This phase may last for years. The patients have allergic rhinitis, nasal polyps, asthma or a combination. Phase 2: eosinophilia in peripheral blood and tissue is typical. For clinical presentation is similar to Loeffler’s syndrome include chronic eosinophilic pneumonia and eosinophilic gastroenteritis. Phase 3: There is a potentially life threatening vasculitis developed. Systemic symptoms (eg. As fever, fatigue, weight loss, fatigue) are often at this stage. However, the phases do not necessarily follow each other, and the time interval between them varies greatly. Various organs and systems are affected: Respiratory: Asthma, often with adult onset, occurs in most patients and tends to heavy and kortikosteroidabhängigem course. Sinusitis is often, but not destructive, without severe necrotizing inflammation. Patients are short of breath. Because of the alveolar hemorrhage cough and hemoptysis may be present. Transient patchy pulmonary infiltrates are common. Neurological: Neurological symptoms are very common. Multiple mononeuropathy (mononeuritis multiplex) occurs in up to three-quarters of patients. CNS involvement is rare, but can include hemiparesis, confusion, seizures and coma, with or without cranial nerve palsies or evidence of cerebral infarction. Kutan: the skin is affected in about half of patients. Nodules and papules show up on the extensor surfaces of the extremities. They are caused by extravascular palisading granulomatous lesions with central necrosis. It may develop with or without prominent eosinophilic infiltration purpura or erythematous papules due leukocytoclastic vasculitis. Musculoskeletal: Occasionally joint pain, muscle pain or arthritis may occur, usually during the vasculitic phase. Heart: The cardiac involvement, a major cause of mortality, heart failure includes due to myocarditis and endomyocardial fibrosis, coronary vasculitis (possibly with myocardial infarction), heart valve disease and pericarditis. The predominant histopathological findings are eosinophilic myocarditis. Gastrointestinal: Up to one third of the patients shows gastrointestinal symptoms (. For example, abdominal pain, diarrhea, bleeding, cholecystitis akalkulöse) due to eosinophilic gastroenteritis or mesenteric ischemia due to a vasculitis. Renal: The kidneys are affected less frequently than other vasculitic diseases that are associated with ANCA. Typically, a pauci-immune (with few if any immune complexes), focal segmental necrotizing glomerulonephritis with crescent formation before; eosinophilic or granulomatous inflammation of the kidneys is rare. Cutaneous vasculitis (Lower Extremities) © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/391_leukocytoclastic_vasculitis_slide-9_springer_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – /media/manual/professional/images/391_leukocytoclastic_vasculitis_slide-9_springer_high_de.jpg?la=de&thn=0 ‘, title:’ Cutaneous vasculitis (Lower extremities) ‘description:’ u003Ca id = “v37892952 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eDiese figure shows multiple ecchymosis and purpura on the legs. u003c / p u003e u003c / div u003e ‘credits ‘© Springer Science + Business Media’

Health Life Media Team

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